Low-grade small round cell tumor of the cauda equina with EWSR1-WT1 fusion and indolent clinical course

Nasir Ud Din; Melike Pekmezci; Gohar Javed; Andrew E. Horvai; Zubair Ahmad; Muhammad Faheem; Anthony L. Navarro; Dolores López-Terrada; Arie Perry

doi:10.1016/j.humpath.2014.09.015

Low-grade small round cell tumor of the cauda equina with EWSR1-WT1 fusion and indolent clinical course

Nasir Ud Din, Melike Pekmezci, Gohar Javed, Andrew E. Horvai, Zubair Ahmad, Muhammad Faheem, Anthony L. Navarro, Dolores López-Terrada, Arie Perry

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22 Citations (Scopus)

Abstract

We report a case of a longstanding, large tumor involving spinal nerve roots of the cauda equina. The tumor showed small round cells arranged in nests and cords and immunophenotypic features of a glomus tumor, along with infrequent mitoses and a low Ki-67 labeling index, but exhibited some rosette-like structures, with focal CD99 and Neu-N expression. Subsequent molecular analysis showed the presence of an EWSR1-WT1 gene fusion by fluorescence in situ hybridization, which was confirmed by reverse- transcriptase polymerase chain reaction. To our knowledge, this is the first case reported with EWSR1-WT1 fusion in a small round blue cell tumor with smooth muscle differentiation and an indolent course.

Original language	English
Pages (from-to)	153-158
Number of pages	6
Journal	Human Pathology
Volume	46
Issue number	1
DOIs	https://doi.org/10.1016/j.humpath.2014.09.015
Publication status	Published - 1 Jan 2015

Keywords

Bilateral
Desmoplastic small round cell tumor
EWSR1
EWSR1-WT1
Glomus tumor
Spinal nerves

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10.1016/j.humpath.2014.09.015

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title = "Low-grade small round cell tumor of the cauda equina with EWSR1-WT1 fusion and indolent clinical course",

abstract = "We report a case of a longstanding, large tumor involving spinal nerve roots of the cauda equina. The tumor showed small round cells arranged in nests and cords and immunophenotypic features of a glomus tumor, along with infrequent mitoses and a low Ki-67 labeling index, but exhibited some rosette-like structures, with focal CD99 and Neu-N expression. Subsequent molecular analysis showed the presence of an EWSR1-WT1 gene fusion by fluorescence in situ hybridization, which was confirmed by reverse- transcriptase polymerase chain reaction. To our knowledge, this is the first case reported with EWSR1-WT1 fusion in a small round blue cell tumor with smooth muscle differentiation and an indolent course.",

keywords = "Bilateral, Desmoplastic small round cell tumor, EWSR1, EWSR1-WT1, Glomus tumor, Spinal nerves",

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doi = "10.1016/j.humpath.2014.09.015",

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T1 - Low-grade small round cell tumor of the cauda equina with EWSR1-WT1 fusion and indolent clinical course

AU - Ud Din, Nasir

AU - Pekmezci, Melike

AU - Javed, Gohar

AU - Horvai, Andrew E.

AU - Ahmad, Zubair

AU - Faheem, Muhammad

AU - Navarro, Anthony L.

AU - López-Terrada, Dolores

AU - Perry, Arie

PY - 2015/1/1

Y1 - 2015/1/1

N2 - We report a case of a longstanding, large tumor involving spinal nerve roots of the cauda equina. The tumor showed small round cells arranged in nests and cords and immunophenotypic features of a glomus tumor, along with infrequent mitoses and a low Ki-67 labeling index, but exhibited some rosette-like structures, with focal CD99 and Neu-N expression. Subsequent molecular analysis showed the presence of an EWSR1-WT1 gene fusion by fluorescence in situ hybridization, which was confirmed by reverse- transcriptase polymerase chain reaction. To our knowledge, this is the first case reported with EWSR1-WT1 fusion in a small round blue cell tumor with smooth muscle differentiation and an indolent course.

AB - We report a case of a longstanding, large tumor involving spinal nerve roots of the cauda equina. The tumor showed small round cells arranged in nests and cords and immunophenotypic features of a glomus tumor, along with infrequent mitoses and a low Ki-67 labeling index, but exhibited some rosette-like structures, with focal CD99 and Neu-N expression. Subsequent molecular analysis showed the presence of an EWSR1-WT1 gene fusion by fluorescence in situ hybridization, which was confirmed by reverse- transcriptase polymerase chain reaction. To our knowledge, this is the first case reported with EWSR1-WT1 fusion in a small round blue cell tumor with smooth muscle differentiation and an indolent course.

KW - Bilateral

KW - Desmoplastic small round cell tumor

KW - EWSR1

KW - EWSR1-WT1

KW - Glomus tumor

KW - Spinal nerves

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DO - 10.1016/j.humpath.2014.09.015

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AN - SCOPUS:84915820177

SN - 0046-8177

VL - 46

SP - 153

EP - 158

JO - Human Pathology

JF - Human Pathology

IS - 1

ER -

Low-grade small round cell tumor of the cauda equina with EWSR1-WT1 fusion and indolent clinical course

Abstract

Keywords

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